Poor general condition with sudden muscle weakness or persistent muscle soreness should be thought of as dermatomyositis or lila disease, especially in women and children. Certain skin changes on the face and neck are also signs of this condition.
What is dermatomyositis?
Dermatomyositis is a rheumatic disease in which there is an inflammatory reaction of the skin and transverse striated muscles. It is a rare disease that progresses in phases and can occur in childhood or adulthood. The juvenile form begins at the age of seven to eight years. Both sexes are affected equally often. The adult form of dermatomyositis, on the other hand, affects women much more frequently between the ages of 35 and 44 and between 55 and 60. From an age of 50 years, in addition to the rheumatic muscle disease, tumors in the gastrointestinal area, in the chest as well as in the lungs occur more frequently.
Causes
The cause of dermatomyositis has not yet been determined. However, it is suspected that certain autoimmune diseases are involved in the development process, in which the immune defense is mistakenly directed against the body’s own muscle and skin cells of the affected person, instead of attacking invading bacteria or viruses as usual. As a result, sufferers complain of general muscle weakness and fatigue, and sometimes hair loss. Everyday activities can only be carried out with difficulty. The skin changes appear mainly as swelling of the eyelids and redness of the skin on the face and neck. On the hands, the nail fold is often painfully inflamed and discoloration of the skin and areas of skin that are conspicuous due to their deviating skin thickness are formed. In dermatomyositis, this primarily affects areas of the body that are exposed to light.
Symptoms, complaints, and signs
Dermatomyositis can lead to very severe restrictions in the life and everyday life of the affected person and thus also significantly reduce the patient’s quality of life. First and foremost, those affected by this disease suffer from severe muscle weakness. It is therefore not uncommon for patients to suffer from permanent fatigue and exhaustion, with the result that most sufferers are no longer able to actively participate in everyday life. Likewise, the performance of strenuous physical activities or sports is no longer possible without further ado. Furthermore, there is severe pain in the muscles, even if they have not been subjected to too much strain. In dermatomyositis, the pain resembles sore muscles and significantly restricts the patient in his or her everyday life. This can also lead to movement restrictions, so that some affected persons are dependent on the help of other people in their lives. Complaints can also occur in the face, so that some affected persons suffer from swelling or redness of the skin. The symptoms of dermatomyositis often lead to psychological complaints, with some patients suffering from depression or other psychological upsets. Whether or not there is a decreased life expectancy due to dermatomyositis depends very much on the exact cause of this disease.
Diagnosis
The diagnosis of dermatomyositis is often already made based on muscle symptoms and changes in the patient’s skin and confirmed by laboratory testing of the blood. An elevated creatinine kinase (CK) level and increased inflammatory parameters are characteristic of this disease. Autoantibodies can also be detected during blood analysis. To detect irregularities in the muscle fibers and swelling, the muscles are assessed during an ultrasound examination. Sometimes magnetic resonance imaging (MRI) is also useful for diagnosis, as this imaging procedure can show possible muscle changes at a particularly early stage. To rule out other diseases and thus confirm the diagnosis of dermatomyositis, a skin sample or even a muscle biopsy is taken under local anesthesia. The tissue obtained can then be examined histologically.
Complications
In dermatomyositis, there is a general feeling of illness and weakness. The patient is no longer able to perform physical activities and is thus severely restricted in his or her daily life.Muscle strength also decreases and is usually accompanied by changes in the skin. Those affected suffer from permanent fatigue. This fatigue is not combated even if the patient rests for a long time or sleeps longer. After physical activity, the patient suffers from severe muscle soreness, which in some cases can occur even without activity. The pain usually causes restrictions in movement and thus in everyday life. The face suffers from swelling and redness of the skin, so the patient usually feels uncomfortable and ashamed of these symptoms. It is not possible to treat dermatomyositis directly. However, immunosuppressants are used to prevent reactions of the body. Likewise, the patient needs an adequate supply of vitamins and trace elements. As a rule, it is not advisable for the affected person to spend a long time in the sun without sunscreen. The muscle complaints can be treated during physiotherapy. No further complications occur. However, the patient is severely restricted in his daily life in case of permanent dermatomyositis.
When should one go to the doctor?
If swelling and redness of the skin in the face and neck area are noticed, it is possibly dermatomyositis. A visit to a dermatologist is recommended if the skin changes persist longer than usual and are not due to a clear cause. Risk groups such as people with autoimmune diseases or an existing skin disease should talk to the treating physician in case of the mentioned complaints. If the complaints massively affect the well-being, a medical clarification is necessary in any case. Symptoms such as muscle weakness, fatigue or movement restrictions in particular should be clarified immediately. Anyone who already notices complications such as increasing movement restrictions or intense fatigue should see a doctor immediately and have the cause clarified. Once diagnosed, dermatomyositis can be treated well. However, if left untreated, further complaints can develop that massively reduce well-being. For this reason, it is important to inform the doctor directly in the event of unusual skin changes and muscle complaints. Accompanying physiotherapeutic treatment is recommended. The measures of physiotherapy alleviate the acute discomfort and prevent a recurrence of the disease.
Treatment and therapy
Dermatomyositis is generally not curable. Therefore, treatment of the disease is based on the symptoms of the individual patient and his or her age. Initially, immunosuppressive and anti-inflammatory drugs are used to intervene in the disease process to suppress the inflammatory response and weaken the immune system‘s reaction to the body’s own cells. The anti-inflammatory drugs must be taken in a high dosage at the beginning, but can be reduced after some time, as they cause numerous side effects. In the course of the disease, the dosage must be adjusted more frequently to the patient’s acute symptoms. However, a drug for immunosuppression must be taken permanently to alleviate the symptoms. Osteoporosis may occur as a result of the anti-inflammatory preparations. For prevention, the patient must ensure sufficient intake of vitamin D and calcium. Since this is hardly possible through food, appropriate medication is prescribed. The diet should also be low in salt. Due to the skin changes, attention should also be paid to adequate sun protection. Dermatomyositis progresses in phases. In the case of an acute disease flare-up, the patient must remain on bed rest, but after the symptoms have subsided, he should participate in the maintenance of his muscle functions with physiotherapy and other physiotherapeutic applications.
Outlook and prognosis
Dermatomyositis initially has a chronic disease course that cannot be stopped. The disease cannot be cured with current medical options. The treatment plan is to alleviate the existing symptoms. In addition, the progress of the disease should be delayed as much as possible. In a long-term therapy the rheumatic complaints can be alleviated and the quality of life is considerably improved. The specificity of the disease becomes apparent after a few years. After five to ten years, more than 80% of patients experience a standstill in the progress of the disease.The reasons lie in the therapeutic measures taken or in an unexplained spontaneous remission of the disease. The remaining 20% of sufferers continue to suffer from dermatomyositis after more than ten years and do not experience any recovery. They belong to a risk group that suffers more frequently from sequelae. The prognosis of dermatomyositis worsens as soon as complications arise. If a tumor develops, the patient’s condition may become life-threatening. In a large number of sufferers, a malignant tumor forms in the area of the heart or lungs. Just over 60% of patients survive the first five years after diagnosis. After ten years, just under half of all sufferers do.
Prevention
The development of dermatomyositis cannot be actively prevented. After the diagnosis has been made, however, the patient should definitely take advantage of physiotherapeutic applications to maintain muscle function between the acute phases of the disease in order to counteract a permanent restriction of movement.
Aftercare
In most cases, the options for aftercare for dermatomyositis are severely limited. In this regard, the patient is primarily dependent on the correct examination, diagnosis and treatment of this disease, so that further compilations and complaints are prevented. In any case, early treatment has a positive effect on the further course of the disease and should therefore always be strived for. In most cases, patients with dermatomyositis are dependent on taking immunosuppressants. These should therefore be taken regularly and as prescribed by a physician. In cases of doubt or other ambiguities, a pharmacist or physician should always be consulted to avoid further discrepancies. Many patients are also dependent on taking preparations containing vitamin D, although a healthy lifestyle with a balanced diet can also have a positive effect on the course of dermatomyositis. In general, the affected person should keep to bed rest and therefore also not exert themselves unnecessarily or move around unnecessarily. Stressful situations should therefore also be avoided as far as possible. Rehabilitation measures of physiotherapy become necessary in some cases, although some of the exercises can also be performed in the patient’s own home.
This is what you can do yourself
Sunlight or even artificial UV light can trigger dermatomyositis or make an existing condition worse. Patients should therefore avoid extensive sunbathing, stay outdoors during the midday heat in the summer months if possible, and always use a sunscreen with SPF 25 or higher. During acute dermatomyositis, visits to solariums are also not recommended. The cortisone preparations prescribed by a doctor should be used consistently, despite side effects. Under no circumstances should cortisone therapy simply be discontinued without consulting a physician. Since cortisone promotes the development of osteoporosis, those affected can take countermeasures. It makes sense to take calcium as well as to ensure an adequate supply of vitamin D. Calcium-rich foods can be consumed for this purpose, although the intake of calcium from cow’s milk is now highly controversial. Plant-based milk alternatives enriched with calcium are preferable here. Calcium can also be purchased in the form of effervescent or chewable tablets from pharmacies or health food stores. Vitamin D is produced by the body itself, provided that sufficient sunlight is available. However, since UV rays have a harmful effect in dermatomyositis, dietary supplements should be used here as well. The purple spots on the skin can be covered with the help of a green concealer or, in severe cases, with special camouflage makeup.
